| Morquio's Syndrome Accompanied by Respiratory Failure |
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Jun Hur, Hyeon Jeong Lee, Jeong Hwa Whang, Hee Jin Kim, Chan Yung Kim |
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Derpartment of Pediatrics, Choon Hae Hospital, Pusan, Korea |
| 호흡 부전을 동반한 Morquio증후군 1례 |
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허준, 이현정, 황정화, 김희진, 김찬영 |
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춘해병원 소아과 |
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| Abstract |
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Authors experienced a case of Morquio’s syndrome accompanied by respiratory failure
in a aged fifteen year old boy. Patient showed normal intelligence, but stunted growth with
short trunk and unproportionately large head, pectus carinatum, kyposcoliosis, muscular
weakness, paralysis and genu valgum. No other member of family was affected with this
syndrome.
There were radiological findings of subluxation between first and second cervical
vertevrae, central beaking with thoracolumbar vertebra plana, wine glass appeared pelvis
and shortening of carpal & metacarpal bones of both hands.
There were biochemical findings of large proportion of chondroitin sulfate(87%) on
urinary mucopolysaccharides examination.
Resipratory failure occurred due to compression of cervical spinal cord by subluxation
between first and second cervival vertebra. He was maintanied by ventilator, discharged.
And he died. |
| Key Words:
Morquio’s syndrome, Respiratory failure, Subluxation of cervival vertebra |
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